New treatment guidelines released for a challenging brain disease

When the immune system attacks a child's brain, a devastating illness called Pediatric Acute-onset Neuropsychiatric Syndrome can result. Children with PANS suddenly develop severe obsessive-compulsive symptoms, struggle to eat, and experience a huge uptick in separation anxiety. Physicians think the illness arises from brain inflammation that follows a triggering infection.

The disease is very hard on kids and families. "Parents often describe children as turning into somebody completely different overnight," according to pediatric psychiatrist Margo Thienemann, MD, who co-directs the PANS clinic at Lucile Packard Children's Hospital Stanford.

Recognition of the disease has been slow to catch on; although a handful of scientists have been studying it for a few decades, the Packard Children's clinic was the first in the country devoted to PANS and its companion diagnosis, Pediatric Autoimmune Neuropsychiatric Disorder Associated with Streptococcal Infections (PANDAS), when it opened in 2012.

Many doctors still don't know how to identify or address the disease, and I recently spoke with Thienemann about the first peer-reviewed treatment guidelines for PANS and PANDAS, which she helped to write earlier this year. "We want physicians to take this disease seriously and, even more importantly, we really want them to treat it," she told me. (Thienemann also helped write the first diagnostic guidelines, which were published in 2015.)

In a Q&A story based on our based on our conversation, Thienemann talked about the importance of helping children with both the medical and psychiatric aspects PANS/PANDAS. Treating the disease comes with some unique challenges, she explained:

Unfortunately, a lot of the tools in psychiatry are medications — for OCD, depression, ADHD, tics — that don’t work the same way on an acutely inflamed nervous system as they would on garden-variety, idiopathic childhood mental illnesses. When medications are used, usually for residual symptoms, the guidelines suggest beginning with one-fourth of the typical starting dose of medication and increasing the dose very slowly. There can be complex interactions between medications, and between the medications and other changing variables related to where a child is in the illness and concomitant treatments. It makes treatment complicated.

The scenario many of us learned in school is that two X chromosomes make someone female, and an X and a Y chromosome make someone male. These are simplistic ways of thinking about what is scientifically very complex.